cleidocranial dysplasia (CCD) is a dysmorphic syndrome characterized by delayed ossification of the cranial sutures, clavicular hypoplasia and dental dysplasia. We recently observed a 24-year old male patient with CCD and associated syringomyelia and Chiari I malformation (CMI). Only three cases with such an association had been described. The role of posterior fossa bone dysplasia in the pathogenesis of CMI and syringomyelia is discussed.